Article
Alport syndrome and Pierson syndrome: Diseases of the glomerular basement membrane.
Matrix biology : journal of the International Society for Matrix Biology - 1 Oct 2018
Funk Steven D, Lin Meei-Hua, Miner Jeffrey H
Abstract excerpt
The glomerular basement membrane (GBM) is an important component of the kidney's glomerular filtration barrier. Like all basement membranes, the GBM contains type IV collagen, laminin, nidogen, and heparan sulfate proteoglycan. It is flanked by the podocytes and glomerular endothelial cells that both synthesize it and adhere to it. Mutations that affect the GBM's collagen α3α4α5(IV) components cause Alport...
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